Lymphoma-induced hemophagocytic lymphohistiocytosis (HLH) – case report Case report
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare (especially in adults) syndrome in which hyperinflammation leads to fever, hepatosplenomegaly, pancytopenia and when untreated leads to death. Characteristic laboratory findings include: hyperferritinemia, hypertrigliceridemia, hypofibrinogenemia, hemophagocytosis, low/absent NK-cell activity and high serum sCD25 levels. HLH in adults is in most cases secondary to viral infection (with an Epstein-Barr virus predominacy). A very rare case of T-cell lymphoma-induced HLH in an 80 year old patient is reported. This diagnosis was made when patient was already in critical condition and although after introducing treatment according to the HLH-2004 protocol (etoposide, cyclosporine A, corticosteroids) signs of improvement were observed, the outcome was fatal. Described case shows usefulness of more frequent ferritin concentration measurements in similar patients. Extremely high (> 10 000 μg/l) hyperferritinemia is a hallmark of HLH and may enable control of this syndrome which is 100% fatal without treatment.
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